Skip Header

You are using a version of browser that may not display all the features of this website. Please consider upgrading your browser.

UniProtKB - P07101 (TY3H_HUMAN)

Protein

Tyrosine 3-monooxygenase

Gene

TH

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

Plays an important role in the physiology of adrenergic neurons.

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">Function</a> section describes the catalytic activity of an enzyme, i.e. a chemical reaction that the enzyme catalyzes.<p><a href='/help/catalytic_activity' target='_top'>More...</a></p>Catalytic activityi

<p>This subsection of the ‘Function’ section provides information relevant to cofactors. A cofactor is any non-protein substance required for a protein to be catalytically active. Some cofactors are inorganic, such as the metal atoms zinc, iron, and copper in various oxidation states. Others, such as most vitamins, are organic.<p><a href='/help/cofactor' target='_top'>More...</a></p>Cofactori

Fe2+

<p>This subsection of the ‘Function’ section describes regulatory mechanisms for enzymes, transporters or microbial transcription factors, and reports the components which regulate (by activation or inhibition) the reaction.<p><a href='/help/activity_regulation' target='_top'>More...</a></p>Activity regulationi

Phosphorylation leads to an increase in the catalytic activity.

<p>This subsection of the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section describes the metabolic pathway(s) associated with a protein.<p><a href='/help/pathway' target='_top'>More...</a></p>Pathwayi: dopamine biosynthesis

This protein is involved in step 1 of the subpathway that synthesizes dopamine from L-tyrosine.
Proteins known to be involved in the 2 steps of the subpathway in this organism are:
  1. Tyrosine 3-monooxygenase (TH)
  2. Aromatic-L-amino-acid decarboxylase (DDC)
This subpathway is part of the pathway dopamine biosynthesis, which is itself part of Catecholamine biosynthesis.
View all proteins of this organism that are known to be involved in the subpathway that synthesizes dopamine from L-tyrosine, the pathway dopamine biosynthesis and in Catecholamine biosynthesis.

Sites

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Function’ section indicates at which position the protein binds a given metal ion. The nature of the metal is indicated in the ‘Description’ field.<p><a href='/help/metal' target='_top'>More...</a></p>Metal bindingi361IronBy similarity1
Metal bindingi366IronBy similarity1
Metal bindingi406IronBy similarity1

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

View the complete GO annotation on QuickGO ...

GO - Biological processi

View the complete GO annotation on QuickGO ...

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionMonooxygenase, Oxidoreductase
Biological processCatecholamine biosynthesis, Neurotransmitter biosynthesis
LigandIron, Metal-binding

Enzyme and pathway databases

BRENDA Comprehensive Enzyme Information System

More...BRENDAi		1.14.16.2 2681

Reactome - a knowledgebase of biological pathways and processes

More...Reactomei		R-HSA-209905 Catecholamine biosynthesis

SIGNOR Signaling Network Open Resource

More...SIGNORi		P07101

UniPathway: a resource for the exploration and annotation of metabolic pathways

More...UniPathwayi
UPA00747;UER00733

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Tyrosine 3-monooxygenase (EC:1.14.16.2)
Alternative name(s):
Tyrosine 3-hydroxylase
Short name:
TH
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:TH
Synonyms:TYH
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryota › Metazoa › Chordata › Craniata › Vertebrata › Euteleostomi › Mammalia › Eutheria › Euarchontoglires › Primates › Haplorrhini › Catarrhini › Hominidae › Homo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 11

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...EuPathDBi		HostDB:ENSG00000180176.14

Human Gene Nomenclature Database

More...HGNCi		HGNC:11782 TH

Online Mendelian Inheritance in Man (OMIM)

More...MIMi		191290 gene

neXtProt; the human protein knowledge platform

More...neXtProti		NX_P07101

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Segawa syndrome autosomal recessive (ARSEGS)23 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionA form of DOPA-responsive dystonia presenting in infancy or early childhood. Dystonia is defined by the presence of sustained involuntary muscle contractions, often leading to abnormal postures. Some cases present with parkinsonian symptoms in infancy. Unlike all other forms of dystonia, it is an eminently treatable condition, due to a favorable response to L-DOPA.
See also OMIM:605407
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_072863207C → Y in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072864227D → G in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_014026233R → H in ARSEGS; loss of over 80% of tyrosine hydroxylase activity; shifted substrate specificity from tyrosine to phenylalanine and Dopa. 4 PublicationsCorresponds to variant dbSNP:rs80338892EnsemblClinVar.1
Natural variantiVAR_014027236L → P in ARSEGS; severe parkinsonian symptoms in early infancy; strongly reduced stability and catalytic activity; rare mutation. 3 PublicationsCorresponds to variant dbSNP:rs121917763EnsemblClinVar.1
Natural variantiVAR_072865241A → T in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072866246H → Y in ARSEGS; loss of about 40% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072867247G → S in ARSEGS; loss of about 50% of tyrosine hydroxylase activity; shifted substrate specificity from tyrosine to phenylalanine and Dopa. 2 PublicationsCorresponds to variant dbSNP:rs762304556Ensembl.1
Natural variantiVAR_071715251P → L in ARSEGS. 1 Publication1
Natural variantiVAR_072868259E → G in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_014028276T → P in ARSEGS; parkinsonian symptoms in infancy; no effect on tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs28934581EnsemblClinVar.1
Natural variantiVAR_071716279C → F in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs1273610334Ensembl.1
Natural variantiVAR_072869294G → R in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs755536257EnsemblClinVar.1
Natural variantiVAR_071717296R → Q in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs199961079Ensembl.1
Natural variantiVAR_072870301P → A in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072871309F → S in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_014029314T → M in ARSEGS; parkinsonian symptoms in infancy; loss of about 80% of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs121917764EnsemblClinVar.1
Natural variantiVAR_071718315G → S in ARSEGS. 2 PublicationsCorresponds to variant dbSNP:rs1288483479Ensembl.1
Natural variantiVAR_072872319R → P in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072873328R → W in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs1428589694Ensembl.1
Natural variantiVAR_014030337R → H in ARSEGS; parkinsonian symptoms in infancy; no effect on tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs28934580EnsemblClinVar.1
Natural variantiVAR_072874359C → F in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs121917765EnsemblClinVar.1
Natural variantiVAR_072875375F → L in ARSEGS; loss of over 80% of tyrosine hydroxylase activity; shifted substrate specificity from tyrosine to phenylalanine and Dopa. 2 PublicationsCorresponds to variant dbSNP:rs763198914Ensembl.1
Natural variantiVAR_072876376A → V in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_071719382I → T in ARSEGS. 1 Publication1
Natural variantiVAR_072877385A → V in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs763039181Ensembl.1
Natural variantiVAR_072878387L → M in ARSEGS; no effect on tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072879394I → T in ARSEGS; complete loss of tyrosine hydroxylase activity. 3 Publications1
Natural variantiVAR_072880399T → M in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs1057520384Ensembl.1
Natural variantiVAR_072881408G → R in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs745551241EnsemblClinVar.1
Natural variantiVAR_014031412Q → K in ARSEGS; loss of over 80% of tyrosine hydroxylase activity; reduced affinity for L-tyrosine. 3 PublicationsCorresponds to variant dbSNP:rs121917762EnsemblClinVar.1
Natural variantiVAR_072882414G → R in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 3 PublicationsCorresponds to variant dbSNP:rs370962049Ensembl.1
Natural variantiVAR_071720428G → R in ARSEGS; phenotype with prominent levodopa-responsive myoconus-dystonia (M-D). 2 PublicationsCorresponds to variant dbSNP:rs1264884607Ensembl.1
Natural variantiVAR_072883441R → P in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs367874223Ensembl.1
Natural variantiVAR_072884467S → G in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072885492P → L in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs767635052Ensembl.1
Natural variantiVAR_014032494T → M in ARSEGS; parkinsonian symptoms in infancy; no effect on tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs45471299EnsemblClinVar.1
Natural variantiVAR_072886498D → G in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 4 PublicationsCorresponds to variant dbSNP:rs771351747Ensembl.1
Natural variantiVAR_072887510L → Q in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
May play a role in the pathogenesis of Parkinson disease (PD). A genome-wide copy number variation analysis has identified a 34 kilobase deletion over the TH gene in a PD patient but not in any controls.1 Publication

Keywords - Diseasei

Disease mutation, Dystonia, Parkinson disease, Parkinsonism

Organism-specific databases

DisGeNET

More...DisGeNETi		7054

GeneReviews a resource of expert-authored, peer-reviewed disease descriptions.

More...GeneReviewsi		TH

MalaCards human disease database

More...MalaCardsi		TH
MIMi605407 phenotype

Open Targets

More...OpenTargetsi		ENSG00000180176

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...Orphaneti		101150 Autosomal recessive dopa-responsive dystonia

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...PharmGKBi		PA351

Chemistry databases

ChEMBL database of bioactive drug-like small molecules

More...ChEMBLi		CHEMBL1969

Drug and drug target database

More...DrugBanki		DB00120 L-Phenylalanine
DB00135 L-Tyrosine
DB03552 Meta-Tyrosine
DB00765 Metyrosine
DB00360 Sapropterin

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...BioMutai		TH

Domain mapping of disease mutations (DMDM)

More...DMDMi		239938945

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_00002055611 – 528Tyrosine 3-monooxygenaseAdd BLAST528

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei19Phosphoserine; by CaMK21 Publication1
Modified residuei62PhosphoserineBy similarity1
Modified residuei71Phosphoserine; by CaMK2 and PKA1 Publication1
Modified residuei502PhosphoserineBy similarity1

Keywords - PTMi

Phosphoprotein

Proteomic databases

jPOST - Japan Proteome Standard Repository/Database

More...jPOSTi		P07101

PaxDb, a database of protein abundance averages across all three domains of life

More...PaxDbi		P07101

PeptideAtlas

More...PeptideAtlasi		P07101

PRoteomics IDEntifications database

More...PRIDEi		P07101

ProteomicsDB human proteome resource

More...ProteomicsDBi		51950
51951 [P07101-2]
51952 [P07101-3]
51953 [P07101-4]

PTM databases

iPTMnet integrated resource for PTMs in systems biology context

More...iPTMneti		P07101

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...PhosphoSitePlusi		P07101

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Mainly expressed in the brain and adrenal glands.

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...Bgeei		ENSG00000180176 Expressed in 85 organ(s), highest expression level in substantia nigra

CleanEx database of gene expression profiles

More...CleanExi		HS_TH

ExpressionAtlas, Differential and Baseline Expression

More...ExpressionAtlasi		P07101 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

More...Genevisiblei		P07101 HS

Organism-specific databases

Human Protein Atlas

More...HPAi		CAB002522
CAB072340
HPA013768
HPA061003

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

GO - Molecular functioni

View the complete GO annotation on QuickGO ...

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...BioGridi		112912, 17 interactors

The Eukaryotic Linear Motif resource for Functional Sites in Proteins

More...ELMi		P07101

Protein interaction database and analysis system

More...IntActi		P07101, 14 interactors

Molecular INTeraction database

More...MINTi		P07101

STRING: functional protein association networks

More...STRINGi		9606.ENSP00000370571

Chemistry databases

BindingDB database of measured binding affinities

More...BindingDBi		P07101

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

Secondary structure

1528
Legend: HelixTurnBeta strandPDB Structure known for this area
Show more details

3D structure databases

Select the link destinations:

Protein Data Bank Europe

More...PDBei

Protein Data Bank RCSB

More...RCSB PDBi

Protein Data Bank Japan

More...PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2XSNX-ray2.68A/B/C/D193-528[»]
4J6SX-ray3.08E/F/G/H1-74[»]

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

More...ProteinModelPortali		P07101

SWISS-MODEL Repository - a database of annotated 3D protein structure models

More...SMRi		P07101

Database of comparative protein structure models

More...ModBasei		Search...

MobiDB: a database of protein disorder and mobility annotations

More...MobiDBi		Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Compositional bias

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes the position of regions of compositional bias within the protein and the particular amino acids that are over-represented within those regions.<p><a href='/help/compbias' target='_top'>More...</a></p>Compositional biasi85 – 90Poly-Ala6

<p>This subsection of the ‘Family and domains’ section provides information about the sequence similarity with other proteins.<p><a href='/help/sequence_similarities' target='_top'>More...</a></p>Sequence similaritiesi

Belongs to the biopterin-dependent aromatic amino acid hydroxylase family.Curated

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...eggNOGi		KOG3820 Eukaryota
COG3186 LUCA

Ensembl GeneTree

More...GeneTreei		ENSGT00940000155711

The HOGENOM Database of Homologous Genes from Fully Sequenced Organisms

More...HOGENOMi		HOG000233373

The HOVERGEN Database of Homologous Vertebrate Genes

More...HOVERGENi		HBG006841

InParanoid: Eukaryotic Ortholog Groups

More...InParanoidi		P07101

KEGG Orthology (KO)

More...KOi		K00501

Identification of Orthologs from Complete Genome Data

More...OMAi		ISVKAPW

Database of Orthologous Groups

More...OrthoDBi		614557at2759

Database for complete collections of gene phylogenies

More...PhylomeDBi		P07101

TreeFam database of animal gene trees

More...TreeFami		TF313327

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

More...Gene3Di		1.10.800.10, 1 hit

Integrated resource of protein families, domains and functional sites

More...InterProi		View protein in InterPro
IPR001273 ArAA_hydroxylase
IPR018301 ArAA_hydroxylase_Fe/CU_BS
IPR036951 ArAA_hydroxylase_sf
IPR036329 Aro-AA_hydroxylase_C_sf
IPR019774 Aromatic-AA_hydroxylase_C
IPR005962 Tyr_3_mOase
IPR019773 Tyrosine_3-monooxygenase-like
IPR021164 Tyrosine_hydroxylase_CS

The PANTHER Classification System

More...PANTHERi		PTHR11473 PTHR11473, 1 hit

Pfam protein domain database

More...Pfami		View protein in Pfam
PF00351 Biopterin_H, 1 hit
PF12549 TOH_N, 3 hits

PIRSF; a whole-protein classification database

More...PIRSFi		PIRSF000336 TH, 1 hit

Protein Motif fingerprint database; a protein domain database

More...PRINTSi		PR00372 FYWHYDRXLASE

Superfamily database of structural and functional annotation

More...SUPFAMi		SSF56534 SSF56534, 1 hit

TIGRFAMs; a protein family database

More...TIGRFAMsi		TIGR01269 Tyr_3_monoox, 1 hit

PROSITE; a protein domain and family database

More...PROSITEi		View protein in PROSITE
PS00367 BH4_AAA_HYDROXYL_1, 1 hit
PS51410 BH4_AAA_HYDROXYL_2, 1 hit

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequences (6+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

This entry describes 6 <p>This subsection of the ‘Sequence’ section lists the alternative protein sequences (isoforms) that can be generated from the same gene by a single or by the combination of up to four biological events (alternative promoter usage, alternative splicing, alternative initiation and ribosomal frameshifting). Additionally, this section gives relevant information on each alternative protein isoform.<p><a href='/help/alternative_products' target='_top'>More...</a></p> isoformsi produced by alternative splicing. AlignAdd to basket
Note: TH transcripts lacking exons 8 and 9 present concomitant splicing in exons 1b and 2.

This entry has 6 described isoforms and 4 potential isoforms that are computationally mapped.Show allAlign All

Isoform 3 (identifier: P07101-1) [UniParc]FASTAAdd to basket
Also known as: TH type 4

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide
        10         20         30         40         50
MPTPDATTPQ AKGFRRAVSE LDAKQAEAIM VRGQGAPGPS LTGSPWPGTA 
        60         70         80         90        100
APAASYTPTP RSPRFIGRRQ SLIEDARKER EAAVAAAAAA VPSEPGDPLE 
       110        120        130        140        150
AVAFEEKEGK AVLNLLFSPR ATKPSALSRA VKVFETFEAK IHHLETRPAQ 
       160        170        180        190        200
RPRAGGPHLE YFVRLEVRRG DLAALLSGVR QVSEDVRSPA GPKVPWFPRK 
       210        220        230        240        250
VSELDKCHHL VTKFDPDLDL DHPGFSDQVY RQRRKLIAEI AFQYRHGDPI 
       260        270        280        290        300
PRVEYTAEEI ATWKEVYTTL KGLYATHACG EHLEAFALLE RFSGYREDNI 
       310        320        330        340        350
PQLEDVSRFL KERTGFQLRP VAGLLSARDF LASLAFRVFQ CTQYIRHASS 
       360        370        380        390        400
PMHSPEPDCC HELLGHVPML ADRTFAQFSQ DIGLASLGAS DEEIEKLSTL 
       410        420        430        440        450
YWFTVEFGLC KQNGEVKAYG AGLLSSYGEL LHCLSEEPEI RAFDPEAAAV 
       460        470        480        490        500
QPYQDQTYQS VYFVSESFSD AKDKLRSYAS RIQRPFSVKF DPYTLAIDVL 
       510        520 
DSPQAVRRSL EGVQDELDTL AHALSAIG
Length:528
Mass (Da):58,600
Last modified:June 16, 2009 - v5
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i31D2D49955ACF070
GO
Isoform 1 (identifier: P07101-2) [UniParc]FASTAAdd to basket
Also known as: TH type 3

The sequence of this isoform differs from the canonical sequence as follows:
     31-34: Missing.

Show »
Length:524
Mass (Da):58,160
Checksum:i708422BBD3304A6C
GO
Isoform 2 (identifier: P07101-3) [UniParc]FASTAAdd to basket
Also known as: HTH-1, hTH-Delta1b,2, TH type 1

The sequence of this isoform differs from the canonical sequence as follows:
     31-61: Missing.

Show »
Length:497
Mass (Da):55,612
Checksum:i6CB8EDC9C4874288
GO
Isoform 4 (identifier: P07101-4) [UniParc]FASTAAdd to basket
Also known as: hTH-Delta2, TH type 2

The sequence of this isoform differs from the canonical sequence as follows:
     35-61: Missing.

Show »
Length:501
Mass (Da):56,052
Checksum:iB614295B9CB2921F
GO
Isoform 5 (identifier: P07101-5) [UniParc]FASTAAdd to basket
Also known as: hTH-Delta2,8,9

The sequence of this isoform differs from the canonical sequence as follows:
     35-61: Missing.
     264-357: Missing.

Note: Lacks catalytic activity.
Show »
Length:407
Mass (Da):45,338
Checksum:i71FB6BA8A6061F44
GO
Isoform 6 (identifier: P07101-6) [UniParc]FASTAAdd to basket
Also known as: hTH-Delta1b,2,8,9

The sequence of this isoform differs from the canonical sequence as follows:
     31-61: Missing.
     264-357: Missing.

Note: Lacks catalytic activity.
Show »
Length:403
Mass (Da):44,898
Checksum:iDAD3F18191575F99
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
H0Y670H0Y670_HUMAN
Tyrosine 3-monooxygenase
TH
45Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H0Y677H0Y677_HUMAN
Tyrosine 3-monooxygenase
TH
116Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
E7EQI0E7EQI0_HUMAN
Tyrosine 3-monooxygenase
TH
48Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
F8W8M5F8W8M5_HUMAN
Tyrosine 3-monooxygenase
TH
44Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence AAA61173 differs from that shown. Reason: Erroneous gene model prediction.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti373R → H in AAI43615 (PubMed:15489334).Curated1
Sequence conflicti401Y → S in AAA61179 (PubMed:2887169).Curated1
Sequence conflicti401Y → S in CAA28908 (PubMed:2882428).Curated1
Sequence conflicti401Y → S in CAA68472 (PubMed:2888085).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_07286219S → C Found in a patient with ARSEGS; unknown pathological significance. 1 PublicationCorresponds to variant dbSNP:rs766704202Ensembl.1
Natural variantiVAR_014025112V → M Common polymorphism. 5 PublicationsCorresponds to variant dbSNP:rs6356EnsemblClinVar.1
Natural variantiVAR_072863207C → Y in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072864227D → G in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_014026233R → H in ARSEGS; loss of over 80% of tyrosine hydroxylase activity; shifted substrate specificity from tyrosine to phenylalanine and Dopa. 4 PublicationsCorresponds to variant dbSNP:rs80338892EnsemblClinVar.1
Natural variantiVAR_014027236L → P in ARSEGS; severe parkinsonian symptoms in early infancy; strongly reduced stability and catalytic activity; rare mutation. 3 PublicationsCorresponds to variant dbSNP:rs121917763EnsemblClinVar.1
Natural variantiVAR_072865241A → T in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072866246H → Y in ARSEGS; loss of about 40% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072867247G → S in ARSEGS; loss of about 50% of tyrosine hydroxylase activity; shifted substrate specificity from tyrosine to phenylalanine and Dopa. 2 PublicationsCorresponds to variant dbSNP:rs762304556Ensembl.1
Natural variantiVAR_071715251P → L in ARSEGS. 1 Publication1
Natural variantiVAR_072868259E → G in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_014028276T → P in ARSEGS; parkinsonian symptoms in infancy; no effect on tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs28934581EnsemblClinVar.1
Natural variantiVAR_071716279C → F in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs1273610334Ensembl.1
Natural variantiVAR_072869294G → R in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs755536257EnsemblClinVar.1
Natural variantiVAR_071717296R → Q in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs199961079Ensembl.1
Natural variantiVAR_072870301P → A in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072871309F → S in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_014029314T → M in ARSEGS; parkinsonian symptoms in infancy; loss of about 80% of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs121917764EnsemblClinVar.1
Natural variantiVAR_071718315G → S in ARSEGS. 2 PublicationsCorresponds to variant dbSNP:rs1288483479Ensembl.1
Natural variantiVAR_072872319R → P in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072873328R → W in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs1428589694Ensembl.1
Natural variantiVAR_014030337R → H in ARSEGS; parkinsonian symptoms in infancy; no effect on tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs28934580EnsemblClinVar.1
Natural variantiVAR_072874359C → F in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs121917765EnsemblClinVar.1
Natural variantiVAR_072875375F → L in ARSEGS; loss of over 80% of tyrosine hydroxylase activity; shifted substrate specificity from tyrosine to phenylalanine and Dopa. 2 PublicationsCorresponds to variant dbSNP:rs763198914Ensembl.1
Natural variantiVAR_072876376A → V in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_071719382I → T in ARSEGS. 1 Publication1
Natural variantiVAR_072877385A → V in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs763039181Ensembl.1
Natural variantiVAR_072878387L → M in ARSEGS; no effect on tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072879394I → T in ARSEGS; complete loss of tyrosine hydroxylase activity. 3 Publications1
Natural variantiVAR_072880399T → M in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs1057520384Ensembl.1
Natural variantiVAR_072881408G → R in ARSEGS. 1 PublicationCorresponds to variant dbSNP:rs745551241EnsemblClinVar.1
Natural variantiVAR_014031412Q → K in ARSEGS; loss of over 80% of tyrosine hydroxylase activity; reduced affinity for L-tyrosine. 3 PublicationsCorresponds to variant dbSNP:rs121917762EnsemblClinVar.1
Natural variantiVAR_072882414G → R in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 3 PublicationsCorresponds to variant dbSNP:rs370962049Ensembl.1
Natural variantiVAR_071720428G → R in ARSEGS; phenotype with prominent levodopa-responsive myoconus-dystonia (M-D). 2 PublicationsCorresponds to variant dbSNP:rs1264884607Ensembl.1
Natural variantiVAR_072883441R → P in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs367874223Ensembl.1
Natural variantiVAR_072884467S → G in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 2 Publications1
Natural variantiVAR_072885492P → L in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs767635052Ensembl.1
Natural variantiVAR_014032494T → M in ARSEGS; parkinsonian symptoms in infancy; no effect on tyrosine hydroxylase activity. 2 PublicationsCorresponds to variant dbSNP:rs45471299EnsemblClinVar.1
Natural variantiVAR_072886498D → G in ARSEGS; loss of over 80% of tyrosine hydroxylase activity. 4 PublicationsCorresponds to variant dbSNP:rs771351747Ensembl.1
Natural variantiVAR_014033499V → M1 PublicationCorresponds to variant dbSNP:rs1800033EnsemblClinVar.1
Natural variantiVAR_072887510L → Q in ARSEGS; complete loss of tyrosine hydroxylase activity. 2 Publications1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes the sequence of naturally occurring alternative protein isoform(s). The changes in the amino acid sequence may be due to alternative splicing, alternative promoter usage, alternative initiation, or ribosomal frameshifting. The information stored in this subsection is used to automatically construct alternative protein sequence(s) for display.<p><a href='/help/var_seq' target='_top'>More...</a></p>Alternative sequenceiVSP_00054431 – 61Missing in isoform 2 and isoform 6. 3 PublicationsAdd BLAST31
Alternative sequenceiVSP_00054331 – 34Missing in isoform 1. 1 Publication4
Alternative sequenceiVSP_00054135 – 61Missing in isoform 4 and isoform 5. 2 PublicationsAdd BLAST27
Alternative sequenceiVSP_054338264 – 357Missing in isoform 5 and isoform 6. 1 PublicationAdd BLAST94

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

More...EMBLi

GenBank nucleotide sequence database

More...GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...DDBJi
Links Updated
M17589 mRNA Translation: AAA61179.1
X05290 mRNA Translation: CAA28908.1
Y00414 mRNA Translation: CAA68472.1
DQ677336 mRNA Translation: ABG73364.1
DQ677337 mRNA Translation: ABG73365.1
AC132217 Genomic DNA No translation available.
M24791, M24787, M24789 Genomic DNA Translation: AAA61173.1 Sequence problems.
CH471158 Genomic DNA Translation: EAX02493.1
BC104967 mRNA Translation: AAI04968.1
BC143611 mRNA Translation: AAI43612.1
BC143614 mRNA Translation: AAI43615.1
M24791, M24787 Genomic DNA Translation: AAA61170.1
M20911 mRNA Translation: AAA61167.1

The Consensus CDS (CCDS) project

More...CCDSi		CCDS31338.1 [P07101-2]
CCDS7730.1 [P07101-3]
CCDS7731.1 [P07101-1]

Protein sequence database of the Protein Information Resource

More...PIRi		A30002 WHHUY4

NCBI Reference Sequences

More...RefSeqi		NP_000351.2, NM_000360.3 [P07101-3]
NP_954986.2, NM_199292.2 [P07101-1]
NP_954987.2, NM_199293.2 [P07101-2]
XP_011518637.1, XM_011520335.2 [P07101-4]

UniGene gene-oriented nucleotide sequence clusters

More...UniGenei		Hs.435609

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...Ensembli		ENST00000333684; ENSP00000328814; ENSG00000180176 [P07101-6]
ENST00000352909; ENSP00000325951; ENSG00000180176 [P07101-3]
ENST00000381175; ENSP00000370567; ENSG00000180176 [P07101-2]
ENST00000381178; ENSP00000370571; ENSG00000180176 [P07101-1]

Database of genes from NCBI RefSeq genomes

More...GeneIDi		7054

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...KEGGi		hsa:7054

UCSC genome browser

More...UCSCi		uc001lvp.3 human [P07101-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

<p>This subsection of the <a href="http://www.uniprot.org/manual/cross_references_section">Cross-references</a> section provides links to various web resources that are relevant for a specific protein.<p><a href='/help/web_resource' target='_top'>More...</a></p>Web resourcesi

Wikipedia

Tyrosine hydroxylase entry

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
M17589 mRNA Translation: AAA61179.1
X05290 mRNA Translation: CAA28908.1
Y00414 mRNA Translation: CAA68472.1
DQ677336 mRNA Translation: ABG73364.1
DQ677337 mRNA Translation: ABG73365.1
AC132217 Genomic DNA No translation available.
M24791, M24787, M24789 Genomic DNA Translation: AAA61173.1 Sequence problems.
CH471158 Genomic DNA Translation: EAX02493.1
BC104967 mRNA Translation: AAI04968.1
BC143611 mRNA Translation: AAI43612.1
BC143614 mRNA Translation: AAI43615.1
M24791, M24787 Genomic DNA Translation: AAA61170.1
M20911 mRNA Translation: AAA61167.1
CCDSiCCDS31338.1 [P07101-2]
CCDS7730.1 [P07101-3]
CCDS7731.1 [P07101-1]
PIRiA30002 WHHUY4
RefSeqiNP_000351.2, NM_000360.3 [P07101-3]
NP_954986.2, NM_199292.2 [P07101-1]
NP_954987.2, NM_199293.2 [P07101-2]
XP_011518637.1, XM_011520335.2 [P07101-4]
UniGeneiHs.435609

3D structure databases

Select the link destinations:
PDBei
RCSB PDBi
PDBji
Links Updated
PDB entryMethodResolution (Å)ChainPositionsPDBsum
2XSNX-ray2.68A/B/C/D193-528[»]
4J6SX-ray3.08E/F/G/H1-74[»]
ProteinModelPortaliP07101
SMRiP07101
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi112912, 17 interactors
ELMiP07101
IntActiP07101, 14 interactors
MINTiP07101
STRINGi9606.ENSP00000370571

Chemistry databases

BindingDBiP07101
ChEMBLiCHEMBL1969
DrugBankiDB00120 L-Phenylalanine
DB00135 L-Tyrosine
DB03552 Meta-Tyrosine
DB00765 Metyrosine
DB00360 Sapropterin

PTM databases

iPTMnetiP07101
PhosphoSitePlusiP07101

Polymorphism and mutation databases

BioMutaiTH
DMDMi239938945

Proteomic databases

jPOSTiP07101
PaxDbiP07101
PeptideAtlasiP07101
PRIDEiP07101
ProteomicsDBi51950
51951 [P07101-2]
51952 [P07101-3]
51953 [P07101-4]

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000333684; ENSP00000328814; ENSG00000180176 [P07101-6]
ENST00000352909; ENSP00000325951; ENSG00000180176 [P07101-3]
ENST00000381175; ENSP00000370567; ENSG00000180176 [P07101-2]
ENST00000381178; ENSP00000370571; ENSG00000180176 [P07101-1]
GeneIDi7054
KEGGihsa:7054
UCSCiuc001lvp.3 human [P07101-1]

Organism-specific databases

Comparative Toxicogenomics Database

More...CTDi		7054
DisGeNETi7054
EuPathDBiHostDB:ENSG00000180176.14

GeneCards: human genes, protein and diseases

More...GeneCardsi		TH
GeneReviewsiTH

H-Invitational Database, human transcriptome db

More...H-InvDBi		HIX0035928
HGNCiHGNC:11782 TH
HPAiCAB002522
CAB072340
HPA013768
HPA061003
MalaCardsiTH
MIMi191290 gene
605407 phenotype
neXtProtiNX_P07101
OpenTargetsiENSG00000180176
Orphaneti101150 Autosomal recessive dopa-responsive dystonia
PharmGKBiPA351

GenAtlas: human gene database

More...GenAtlasi		Search...

Phylogenomic databases

eggNOGiKOG3820 Eukaryota
COG3186 LUCA
GeneTreeiENSGT00940000155711
HOGENOMiHOG000233373
HOVERGENiHBG006841
InParanoidiP07101
KOiK00501
OMAiISVKAPW
OrthoDBi614557at2759
PhylomeDBiP07101
TreeFamiTF313327

Enzyme and pathway databases

UniPathwayi
UPA00747;UER00733

BRENDAi1.14.16.2 2681
ReactomeiR-HSA-209905 Catecholamine biosynthesis
SIGNORiP07101

Miscellaneous databases

The Gene Wiki collection of pages on human genes and proteins

More...GeneWikii		Tyrosine_hydroxylase

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...GenomeRNAii		7054

Protein Ontology

More...PROi		PR:P07101

The Stanford Online Universal Resource for Clones and ESTs

More...SOURCEi		Search...

Gene expression databases

BgeeiENSG00000180176 Expressed in 85 organ(s), highest expression level in substantia nigra
CleanExiHS_TH
ExpressionAtlasiP07101 baseline and differential
GenevisibleiP07101 HS

Family and domain databases

Gene3Di1.10.800.10, 1 hit
InterProiView protein in InterPro
IPR001273 ArAA_hydroxylase
IPR018301 ArAA_hydroxylase_Fe/CU_BS
IPR036951 ArAA_hydroxylase_sf
IPR036329 Aro-AA_hydroxylase_C_sf
IPR019774 Aromatic-AA_hydroxylase_C
IPR005962 Tyr_3_mOase
IPR019773 Tyrosine_3-monooxygenase-like
IPR021164 Tyrosine_hydroxylase_CS
PANTHERiPTHR11473 PTHR11473, 1 hit
PfamiView protein in Pfam
PF00351 Biopterin_H, 1 hit
PF12549 TOH_N, 3 hits
PIRSFiPIRSF000336 TH, 1 hit
PRINTSiPR00372 FYWHYDRXLASE
SUPFAMiSSF56534 SSF56534, 1 hit
TIGRFAMsiTIGR01269 Tyr_3_monoox, 1 hit
PROSITEiView protein in PROSITE
PS00367 BH4_AAA_HYDROXYL_1, 1 hit
PS51410 BH4_AAA_HYDROXYL_2, 1 hit

ProtoNet; Automatic hierarchical classification of proteins

More...ProtoNeti		Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiTY3H_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P07101
Secondary accession number(s): B7ZL70
, B7ZL73, Q0PWM2, Q0PWM3, Q15585, Q15588, Q15589, Q2M3B4
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: April 1, 1988
Last sequence update: June 16, 2009
Last modified: January 16, 2019
This is version 210 of the entry and version 5 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

3D-structure, Complete proteome, Reference proteome

Documents

  1. SIMILARITY comments
    Index of protein domains and families
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. PATHWAY comments
    Index of metabolic and biosynthesis pathways
  6. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  7. Human chromosome 11
    Human chromosome 11: entries, gene names and cross-references to MIM
UniProt is an ELIXIR core data resource
Main funding by: National Institutes of Health

We'd like to inform you that we have updated our Privacy Notice to comply with Europe’s new General Data Protection Regulation (GDPR) that applies since 25 May 2018.

Do not show this banner again